A specific integrin receptor, integrin α1β1, plays a role in the progression of interstitial disease associated with Alport syndrome. This effect was illustrated by crossing the Alport mouse with a knockout mouse for the integrin α1 gene (Cosgrove et al., Am. J. Path., 157, 1649-1659 (2000)). The integrin knockout mutation has no obvious effect on renal development or function in normal mice, even though it is widely expressed in the kidney (Gardner et al., Dev. Biol. 175, 301-313 (1996)). When the α1 integrin mutation was added to the genetic background of the Alport mouse, however, both glomerular and tubulointerstitial disease were markedly attenuated. Attenuation of the glomerular pathogenesis was linked to the effect on mesangial expansion and the deposition of mesangial laminins in the GBM (Cosgrove et al., Am. J. Path., 157, 1649-1659 (2000)). The effect of the α1 integrin null mutation on tubulointerstitial disease, however, was less clear.